Saffy's BMT

T+365. Signing Off.

2005-12-22T20:35:00.000Z

Well, one year on - almost to the day - and we have been signed off by the BMT unit.

When we started this process by Saffy being recommended for a transplant and then starting the search for a donor about 18 months ago, we had little idea what we were letting ourselves in for. We had only an outline idea of what we were letting our little girl in for.
We were convinced that a BMT was our only hope of giving Saff a fighting chance of something like a 'normal' life. The odds were good - around 90% of BMT kids survive the procedure and many of the 10% are those poor kids whose conditions (including cancers and auto-immune diseases) had weakened them already.
Over the weekshowever, it's fair to say that the 10% started to haunt us. Living with the prospect that the worst might actually happen had several effects on us. We prepared like mad - emotionally, practically - we thought about what Saffy would need, what her brother Reuben would need. Who could help with what, who would need what. In all of these preparations our ISMRD friends were immensely helpful and knowledgeable. We made arrangements about work and childcare and my mother agreed to be our 3rd carer. We noticed somewhere along the way that we had stopped being interested in some of the things we used to do that somehow just didn't seem very interesting any more. We probably drank too much.
And then we were in GOSH and life had its own momentum and intensity. We worked at everything we could, learning as much as we could from everyone - medical and other staff at the hospital, other parents, other kids. I won't forget some of the most profound lessons. In a sense, this was the easiest part - it was intense, there was always something that needed doing. We work in deadline-driven jobs so this part played to our strengths. It was all about Saffy (and Reuben) and we did not have too much time to sit and think. Doing the blog every night was therapeutic too.
But, above all, we were very, very lucky. We were well-prepared and expecting infections, rejection, extreme reactions to medication. In the event, none of these turned out to be as bad as we had feared and Saffy was an absolute delight. Her anxieties were there. We saw them for example in her developing a fear of clowns (called coulrophobia, if you're interested) whilst in hospital - but she found the good in every day and every situation.
And now, here we are. BMT officially over. It has been the single defining episode of our lives so far. For Saffy, of course, it is her chance to develop. Her alpha -mannosidase level is 77 on a scale that runs to 520, which puts her at the low end of carrier level. This could go up, but to be honest, our doctors have no idea. What it means to her development will unfold in the years to come. Her doctors, teachers, psychologists, occupational therapist, speech therapists, ophthalmologist, spinal consultant, audiologist, dentist and the rest of the team will monitor and help her.
As for the rest of us, Reuben carries on pretty much as before. We consider this a victory. I am not the same, largely through learning something about myself and my priorities in life. I won't bore you with all of that. Sonja having given up her job to care for our kids through Saffy's BMT is applying to become a teacher. The plan is that this gives her more time to spend with the kids and, although she has not said so, I suspect the opportunity to help and nurture children in some way has more of a magnetic pull now than maybe it would have done before. Getting onto a course will be a new challenge, so fingers crossed.
Before we sign off and complete this blog, which we really hope will be of some help to people starting on the same road in the future, we want to thank all our friends and family - both those we knew before and those we have discovered during the last year and a half. Because things have turned out well, what has happened is that many of our relationships have been strengthened and we have found others who have entered the Woolley Family Hall Of Fame. This isn't Oscar night and I am no Gwyneth Paltrow, so we'll leave it there, but because of the people around us, we believe we are luckier than most.
Thank you.
All our love,
Martin, Sonja, Reuben and Saffron Woolley.

T+208. A trip down memory lane...

2005-07-05T21:12:00.000Z

I can't believe that it's been THAT long since we put an entry up. I mean, I know it was a long time, but....

This is for whoever's reading this in the future, as there's no one who can still be checking for an entry after 95 days of not seeing one.

It seems like a lifetime ago that those entries were coming thick and fast. Since then, it's been like an uneasy limbo. I'm working, Sonja can't think about getting out to work and Saffy can't think about playgroup as every last aspect of our near future is governed by those weekly lymphocyte readings. Saff is doing really well, but it seems that lymphocytes after alpha-mannosidosis BMTs just don't come back quickly. About the same speed as a glacier. We know how lucky we are - we really do - but it just isn't possible to feel lucky all day for weeks and weeks.

Among other things, this has been a voyage of self-discovery for us, and we now know that our patience has limits and that we are better suited temperamentally to the parts of the process that involve immediate action.

So, when Saff developed her first line infection three days ago, we were able to get back to what we do best and react to an evolving situation. To get to 205 days past transplant without having a line infection may be a record - many of our contemporaries in Fox Ward needed all their fingers and toes to count theirs after much less time. Anyway, we hadn't seen one, so it came as something of a shock...

We were actually at the UK MPS Society conference, picking up hints on trigger finger (which Saff is just starting to get) and biomarkers (biochemical indicators of disease), when we came out of the last session, turned on the mobile phones and got a message from my dad that Saffy had a temperature of 39.5 and was on her way into hospital (GOSH organised everything so that she could go into our local hospital). By the time Son and I arrived (in about an hour Northampton - London, hope we didn't pass any speed cameras) Saff was over 40 degrees, fitting, frothing at the mouth and apparently awake, yet unconscious and unresponsive. Pretty scary, but bringing her temerature down brought her back - suggesting that this had been a febrile convulsion.

GOSH took no chances and ordered a head CT scan to check for abcesses on the brain, a lumbar puncture to check for meningitis and a chest x-ray. Saff's temperature was 'spiking' - coming down with paracetemol and ibuprofen, then shooting up as it wore off. She was put on antibiotics to cover all major potential problems - particularly meningitis. Her lymphocutes dropped to 0.15 from aroung 0.8 a few days before. She was clearly ill, but, as ever, cheerful as she could be. One by one, the really nasty things got ruled out, thank goodness.

Anyway, within a day the hospital had identified Staphylococcus and Saff had another antibiotic added to her regime. We already 'knew' it was from her line as she had gone downhill within minutes of her Hickman lines being flushed - a sign we'd been told to look out for as the bugs get flushed into her bloodstream from the line where they were sitting, causing the sudden, dramatic deterioration we had witnessed. Tests confirmed this later and Saff is doing much, much better now the bacterial infection is being accurately and aggressively treated. We expect her out in a few days - maybe sooner.

Being back in a hospital room and going through all of the old routines brought it all back (probably why I did this blog, too, I suppose). If the infection doesn't get cleared from her line, it may well be taken out a few weeks ahead of schedule and GOSH think her counts will bounce back once she is better.

The medical staff at both hospitals were really great - professional and caring and made us part of the team immediately and a great 'catch' by Saffy's grandparents who did exactly the right thing by calling them as soon as they got concerned.

If all this excitement keeps up, I'll be putting another blog up soon - great to hear from messages that it has found some people in similar positions already and been of some use.

All the best for now

Martin

Last lap? To T+113

2005-03-31T18:19:00.000Z

Is there anybody out there? Couldn't blame you if you had given up on us after so long without an entry, but as several have mentioned, no news tends to be good news and that has been the case with us.

We are at the point now where Saffy's last immunosuppressant drug (Cyclosporin, aka Neoral) is being eliminated by stages. We are down to 0.3 ml twice a day, from a high of 0.55. This drug suppresses any GvHD, so we can expect to see some sign of it as the dose comes down. So far, all we have seen is the intermittent rash we saw in hospital, which appears and disappears after a few minutes. If we can get away with nothing more than that, it will be a miracle, but then, everything has been a bit miraculous so far.

The issues we have been contending with so far have been different to those we were expecting, as Saff has been so well. With me going back to work, Sonja has been pretty much stuck in the house as Saff is not able to be around groups of people. For someone who has been used to working full time, that is a hard transition to make and it has taken quite some getting used to. For my part, I have been working full time for quite a while and taking over on duty from Sonja at weekends. The practical result of all of this is that it takes quite a bit of planning to do anything outside the usual routines and time out of the house for Sonja and me together, or for us to take Reuben out. Don't get me wrong, we still thank our lucky stars each day, it's just that we are not living what you could call an everyday life yet. Between Reuben's diabetic care and Saff's medication there are around 8 entries in our daily medical diary, when they are both at home, so it is a bit of a stop-start existence.

It has been a great help to us all that friends and family are really putting themselves out for us and the messages we continue to get from friends through ISMRD and GOSH reminds us that we're all getting through with each other's support.

Have to go now - it's bathtime and I've got some Hickman lines to wrap in clingfilm....

All the best

Martin

More of the same............... to +84

2005-03-02T09:26:00.000Z

Sorry for the big gap - but it's getting harder to find things to tell you about here in Camp Lucky. Saffy remains happy and healthy. As yet we have no solution to the continued problem with the red cell count but all medical personnel remain remarkably calm and we are doing our best to mimic that state. In fairness, it's not that hard because Saff looks and acts like a girl thats never been ill in her life.

On our most recent trip to GOSH - last week - the BMT consultant said all that worried him about Saff's tumbling Hb levels was his inability to pinpoint the precise cause. He thought her residual A antibodies attacking the donor AB marrow was the most likely explanation. He felt the immature marrow theory would also lead to reduced white cell counts and this hasn't been the case. What is an undisputed fact is that a significant number of post-transplant children suffer from this problem, and it almost invariably corrects itself, regardless of why it exists in the first place! She hasn't had a transfusion for over two weeks now and although her Hb level is low it's taken longer to fall than usual - so fingers crossed.

I have to confess that all this staying at home has given me an acute attack of cabin fever - I find myself looking forward to shopping trips - and I don't even mean for clothes! Still, all in a good cause.

Reuben, Pokemon master supreme, top Hogwart's wizard and family Top Trumps champion, continues life unconcerned by the endless medical interventions needed for him and his little sister - thank goodness.

Thanks to all of you still following our fortunate progress - I'll try and update you a little sooner next time.

Take care

Sonja

Music to our ears..........to + 58

2005-02-04T08:48:00.000Z

I'm writing this entry from our local hospital as Saff has a blood "top-up". Everything continues to go really well.

We were at GOSH yesterday and saw Paul Veys - he's the "grand fromage" of the BMT unit and was really delighted with Saff's progress to date. He confirmed his belief that Saff's falling Hb levels are caused by immature marrow saying the red blood cells are typically the last to stabilise post transplant. He did however test another couple of possibilities to exclude them. The first is caused by the ciclosporin attacking the cells, they're left looking like they've had a huge bite taken from them. Treatment is simply to withdraw the ciclosporin. The second could be Saffy's original B cells attacking the donor's AB cells - this can occur because B blood cells contain A antibodies. If this is occuring they simply wait until all Saffy's residual B cells disappear and she takes on the donor's AB blood group.

Today's transfusion is to boost her levels closer to normal and then watch to see how long it takes to fall. Paul Veys says even if this process (i.e. transfusion followed by falling Hb) continues for a number of months he will remain unconcerned.

We left GOSH and travelled to the local hospital for the transfusion (obviously!). As Saffy had had blood taken 7 times in the last 10 days it stands to reason more would need to be taken to establish what blood should be ordered!! We arrived at 2.30pm, had blood taken at 3pm, and were told at 6.45pm that although the blood had arrived it was the wrong type. Words failed me - well actually they didn't but I think the nurse who had to tell me this wished they had.

So here we are this morning - I'm considerably calmer now the process is almost over. We're going home so Saffy and her nursery teacher Natalie can have fun. Tuesday's "lesson" involved a fantastic shaving foam fight. It's a pleasure to hear them at work!

More soon

Sonja

P.S. All blood results for the tests above were negative - brilliant

Testing Times......to +53

2005-01-30T19:46:00.000Z

Following a hectic week, Saffy is back to feeling well and all the blood tests were negative - the good luck's still with us.

The slump in her Hb level had caused nasty pressure headaches if she bent over (troublesome if you still need your bottom wiped for you) if she jumped (Saffy's favourite way to move around) or if she moved at speed (although it should be said that Saff speed isn't very speedy!). The headaches disappeared a few hours after the transfusion and haven't plagued her since. At least we now have an early warning signal should this happen again.

The local hospital were very thorough in searching for a reason for Saffy's falling iron level, and although it lead to a nervous 24 hours, we've learnt about haemolysing and that it can be treated should Saffy succomb. It occurs in up to 50% of post bone marrow patients and is caused by the compromised immune system. It is treated with steroids. GOSH believe Saffy's level fell simply because her marrow is immature. Her Hb reading only went up to just over 7 after the transfusion - it should be between 11.5 and 14.5 - and although it seems to have temporarily stabilised we're expecting further transfusions.

Saffy's very mild rash seems to have disappeared, the hospital have lowered her cyclosporin levels so perhaps it will make another appearance but we're seeing no other signs of lurking GvHD (said with everything crossed whilst touching wood).

Although still happy to be home, Saffy misses the routine of her old life, she talks constantly of playgroup and the other children at her childminders. The playgroup have been fantastic and are now sending a teacher round for 2 hours every Tuesday and Thursday, to give Saffy a chance to make and colour things with someone who can actually draw. We're continuing to encourage all our friends with children to drop in, but with the good health proviso in the middle of winter our cancellation levels are expectedly high.

Sorting out getting all of the medications, dressings, syringes etc. is a complete pain. It's not that anyone is being obstructive, there are just too many people to talk to in order to get things organised.

I have to say that yet again our daughter's ridiculously sunny outlook is making life much easier for us and herself. Long may she stay the happy little soul that she is.

Thanks to all of you continuing to follow our journey, particularly now it's getting less dramatic (and our reporting has slowed down!)

Take care

Sonja

Deja vu (all over again) Up to T+48

2005-01-25T21:28:00.000Z

So, here I am at home again with Reuben whilst Son is in hospital with Saff. It's all strangely familiar. Don't be concerned, Saffy is in hospital for her flebogamma (immunoglobulin), which she needs intravenously every 3 weeks. She was also anaemic (Hb of 4.2), so she needed a blood transfusion. She is at our local hospital, who now share her care with GOSH, and not every paediatric ward works with Fox ward's speed, so it has turned into an overnight visit. Saff had been complaining of headaches and tummy aches, but had not developed a fever - we are hoping that she will get the boost from the red blood cell transfusion that she did before. No one at Chase Farm (our local) or GOSH is too concerned - sometimes new marrow just does not produce enough on its own at this stage and some extra is needed. To be on the safe side, they are going to run some tests overnight to see why the red blood cell count is dropping. The 3 usual reasons would be an infection (no sign of this), blood leaking somewhere (ditto) or haemolysing, which, as far as we understand, is the body breaking down red blood cells too readily. If it is this is happening, it leaves markers that can be traced and we will find results out tomorrow.

May I apologise that it has been such a long time since you have heard from us. No excuses, we should have let you know before now what has been happening and I hope that not everyone has given up looking out for updates.

I have gone back to work now, so the feeling of limbo between leaving hospital and getting back to a closer approximation of normal life is starting to disappear. We have set up our home and our routines and support networks pretty well. Medication sets the tempo for life. As suggested by experienced friends, we made spreadsheets of all of Saffy's medications, setting out times and doses. Even though Saffy is on the minimum medication for this stage, it is still remarkably easy to make a slight slip and having it all on paper makes a that a lot easier.

As I mentioned, her care is split between GOS and Chase Farm hospitals. Chase Farm send a community nurse every week to change dressings weekly and new NG tube monthly. They also take bloods on which they can do the more usual tests for cell counts and biochemistry. On our clinic visits to GOSH, they take blood for the more specialised tests, which includes cyclosporin levels. Cyclosporin is one of the key drugs at this stage. Its job is to mute any GvHD, which is important, but in doing this, it also suppresses the lymphocyte levels. This is partly why Saff's immune system is still not working at anything like full capacity. The ideal level is around 150 and, according to the blood test results, her dose is adjusted to try and keep it thereabouts. The theory is that she gets minimal GvHD and they will then gradually bring the dose down and tail it off. Fingers crossed, as usual. That approach has worked until now, so we're not changing.

Day-to-day, Saff is still delighted to be at home. She is starting to show signs of feeling the limitations of her life at the moment and of reacting to all the attention she has inevitably got more used to. This is manifesting in demanding more attention, thinking that she is going to be going to school with Reuben the next day and so on. This is really not much, and she is still far more likely to get excited about getting a visit from someone than worrying about what she's not getting. Her glass is always half-full.

Speaking of visits, we can have 3 or 4 people round to the house at one time, so long as everyone is healthy (no coughs or sneezes, no runny noses, no diarrhoea is the mantra). Other people we know have had more stringent rules on being discharged - we can only assume that this is down to how well Saff is, relatively speaking. This makes a lot of difference as it allows everyone - Sonja & Saff in particular - a break from being in each other's company exclusively, as well as giving friends and family the chance to see Saff for the first time in several weeks. Saffy is also allowed out of the house, but only where she can maintain some distance from other people. At this time of year, that means that if we wrap her up in about a dozen layers, we can get out to a park for a run around as they are very sparsely populated. Shops, restaurants, indoor play areas etc are all no-go areas.

I'll leave it there for now, but we will - we promise! - put another entry up in a day or two to let you know what happened with the blood tests. Radio Saffron is very definitely back on the air.

All the best

Martin

T29, T30, T31, T32. Home Sweet Home.

2005-01-09T21:04:00.000Z

After what seems like the longest time one minute, and an amazingly brief time the next, we are now at home. It is an enormous relief to be here. Of course, getting here was the usual Woolley family mini-epic.

At one point I thought Son was going to suffer some mental trauma induced by the scale of the clean-up operation at home whilst I was doing the last shift at Fox Ward. In typical fashion, she set herself very high standards and then met them all, with the result that the house was not only as hygenic as it has ever been by a comfortable margin, but Saffy's room was a pink paradise covered in (left-over Xmas) presents when she arrived. The look on her little face was priceless. Son will freely admit that she could not have got close to getting everything done without the drive, determination and elbow grease of Margaret, her mum, who came down for 3 days from Worcestershire, just to help out.

Saffy is now spending her time going and finding all her favourite things at home and in between time just grinning. She seems really well 'in herself', as they say. Going home seems to have given her another boost, as did the red blood cell transfusion and the white-blood-cell-boosting G-CSF infusion that she had just before leaving hospital. Both counts were a little on the low side and the doctors wanted to give her body a little extra help to cope with the outside world. Or the limited part of it that she will see from inside the house.

We have set up a M*A*S*H*-style field hospital in Palmers Green with syringes, medecines and other paraphernalia around the house. So far, managing the medications has been fine - Son and I supervise each other to make sure we're putting everything we were told into practice. Keeping the house hygenic is a challenge - Reuben and Saffy managed to wee on or near each other and on the floor within about 1 1/2 hours of getting home.

We have a way to go yet - it's only been 48 hours since we got her home - so, we do not want to take anything for granted, but while the hospital stage that we have just completed is still fresh in our minds, we wanted to note a few things that felt like they had worked for us for anyone reading this in the future preparing for their own child's BMT. This all comes with a health warning because neither we nor anybody else knows anything for certain, but for what it's worth, here goes:

Things Outside Our Control:

We had a good match - even though it was an unrelated donor (10 /10 tissue match, later further refined to 11 /12).

Our donor was physically large, meaning that we could get a lot of marrow - the large volume may have had something to do with how quickly Saffy started showing neutrophils post-transplant.

We had a male donor. Male to female transplants are the most desirable. Frankly, we can't remember what the reason is for this, but the outcome tends to be less severe GvHD.

The support we receive from our friends, family and employers is as much as anyone could wish. Seeing other families cope with less has brought that home. Because of this we have been able to devote ourselves to Saffy and Reuben during this time. If we get through this whole process relatively unscathed, this will be part of the reason why.

The team at GOSH. They are absolutely brilliant. Just brilliant.

Things Within Our Control:

All of the things above (plus luck) probably account for over 90% of success in BMTs. Through what we did we tried to squeeze in another few percent difference. What we did was:

Set very high standards of hygiene. We took the cleanliness rules as gospel and wherever we were told 'do this 3 or 4 times a day' we tried to make sure we did it 4 times, not 3. It is quite possible that either Sonja, Erika or I will have obsessive-compulsive disorder in later life.

Think like a germ or 'If I were a rotovirus, where would I hide'. When you're not doing anything else, look around and find spots like door handles, the tops of high shelves etc and get happy with the alcohol wipes.

Find the nurses who have been on the ward the longest and quiz them. Find out everything you can. Nurses with a strict demeanour are the best for this. We loved the strict nurses, because they weren't afraid of hurting feelings, they just said what they thought was best - for example good ways of separating gloves used for different functions to different parts of the room. Sounds anal. It is. But these nurses really knew their stuff and it makes really good sense.

Talk to everyone. The housekeeper can tell you really useful things like where it's safe to get bread in the middle of the night when the frozen, individually-wrapped portions have run out in the special BMT kitchen and you have a girl suddenly demanding toast after 36 hours. The cleaners are sometimes the same ones who prepare rooms for green precautions - they know more about killing germs in your room than you do and they're full of tips.

Be strict with each other. The 3 carers helped by policing each other - it is really easy to forget instructions when you hear so many, so it helped that we reminded each other.

Take short shifts if you can - 2 nights was our ideal when possible. There is a lot to do, it is stressful in different ways and the nights are always broken, so being able to rotate carers regularly is a good idea.

Write everything down. Take notes when you are learning about things. Also, write everything down during the day. You won't be able to keep a note of when she drank what, which cream you applied when, how many times you did the oral care routine and everything else, all in your head.

As well as all of this, try to make the experience as much fun for your child as possible. This might sound strange, but when you're a kid, there can be fun in anything. Saffy enjoyed watching her old favourite videos as much as she wanted (I can kill at the mere sound of the Balamory theme tune now), getting lots of new sticker books and even playing the 'Who can sellotape up most hair from the pillowcase' - a game that you can only play under very few circumstances! Most of all, stay happy yourself so you create a positive atmosphere.

None of this is rocket science. More an extreme form of common sense. Don't feel too daunted - we learned everything from friends with previous experience, but mainly from the staff at the hospital who are used to guiding families through this, and who could, if necessary, take your child from beginning to end of the process without you.

Anyway, enough top tips. We'll let you know how well things are going at home soon.

All the best from a very happy Team Saff.

Martin

PS. I also matched Son's 'Raspberry Nipple' cock-up, when we were getting the last few thngs together before leaving. As I tried to reach a particularly elusive get well card from a high shelf, I over -reached, but managed to grab it. As I turned to leave the room I was almost knocked down by the charge of nurses and doctors arriving within seconds from other patients' rooms. Obviously in over-reaching I had leant on the crash alarm placed out of the way high on the wall and they were arriving to save somebody's life. I was left in no doubt what a medical faux pas I'd made as I walked past that same team on our way out....

T +25, T + 26, T +27, T +28. One foot out of the door.

2005-01-05T22:45:00.000Z

Well, after not letting ourselves get carried away for the last couple of weeks, it seems that we can now definitely (probably) look forward to taking our little girl home on Friday.

There is one more set of test results that we are waiting for (a virus screen). She is not showing any symptoms, so our expectation is that we will get the all-clear. The nagging sense that something has to go wrong sometime might actually be our worst problem at this precise moment.

So we are feverishly making plans for the return home. We are spending money like it is so far out of fashion that we would rather not be seen with it – even by the standard of the last couple of months. Our 3 cats have had to go into temporary accommodation and we have taken the step of having a professional cleaning company come in to get the house ready. Whilst this is considered a positive move rather than strictly necessary, we have taken a no-risk approach so far and who knows whether that has contributed somehow. We certainly don’t, so it’s no-risk all the way as far as we’re concerned. The most important thing to ensure is that the carpets are not left damp as this can encourage fungi.

Sonja is at home with her mum cleaning toys, throwing away junk and generally applying Fox Ward principles. It all makes us feel like this is a new start.

On the medical front, we’re learning how to administer Saffy’s medicines. They are all going down her naso-gastric tube as she was not happy to take them by mouth. Cyclosporin in particular is very oily and unpleasant to taste and Saffy herself suggested that we use her tube. She will be on Acyclovir, Septrin (aka Co-trimoxazole), Cyclosporin and Penicillin – a much lighter regimen than average as she is doing so well.

Using the NG tube is manageable. You have to draw back a little stomach contents first and test its acidity with indicator paper. This determines that the tube is in the right place and hasn’t worked its way into Saff’s lungs or elsewhere, which can happen. Then you give the medicines down the tube and follow up with water to flush all the drugs to her stomach and put the stopper back in. Saff is pretty expert herself and ‘helps’ with much of the procedure.

We will also be getting training on her Hickman line, having a meeting with the senior nurse here and the local team and generally going to school over the next couple of days. More to follow next week at home, I'm sure - we'll let you know after we get there!

It still runs against the grain to say the decision to let us home is finalised, but we hope that the next update or the one after that will be written at home with all 4 of us there. The feeling of relief at having got that far will be huge, albeit mixed with emotion for the families that we leave behind, many of whom are having the kind of very tough times that we were dreading before we came in and, whatever might be around the corner for us, would swap shoes with us if they could.

All the best from Planet Saffron.

Martin

T +21, T +22, T+23. The ‘H’ word

2004-12-31T21:46:00.000Z

Sooner or later, there comes a point in the BMT process when most of the drugs are finished, the counts have stabilised (at over the required 0.5 neutrophil count – she’s dropped today, but still at 0.95) and there are no infections that need active treatment. At this point there is no further need for hospitalization.

For the first time, we are beginning to hear the ‘home’ word discussed (by someone other than us, that is). If Saffy can stay free of further infection and we do not get any serious GvHD symptoms, we could be out of here in a week. I have spoken to too many parents who were at exactly this stage some time ago, and are still here, to take anything for granted, but it’s exciting nonetheless.

So, over the next few days, the transition will start from hospital to home care. Saffy has already moved from the intravenous form of Cyclosporin, to the oral version, and her vitamin K will stop when she is discharged, so there is nothing else that regularly needs to go down her Hickman line. The next steps are to train us up to be able to administer her drugs at home and to change to timings of her doses so that we do not have to maintain the current through-the-night schedule.

The possibility of acute GvHD still remains until about day 100, which is a black cloud that will follow us around until mid-March. Acute GvHD comes in 4 levels of intensity. Level 1 is unpleasant and needs careful treatment, level 4 is not something I ever want to experience. No one knows for sure what the determining factors are behind whether you get GvHD. It seems strange to me as a lay-person that ‘rejection’ can happen so late in the process – after all, if the marrow is going to react against the host, why doesn’t it do it straight away? The answer lies somewhere in the complex biochemistry that occurs during engraftment. Needless to say, there’s a bit more to it than my Janet’n’John understanding of bone marrow can explain. The fact is we’re not out of the woods for another 75-odd days.

So, now we’re looking at a new phase in the treatment, there’s more to think about. Our house needs to be a lot cleaner that it has been up until now (professional help required), the cats (we have 3) need temporary re-homing, we need to establish workable new routines to cope with having a child at home effectively on yellow precautions. On top of this, we think that this will be the time when we most need to make sure that we are helping Reuben cope with the process. Up to now, he has probably quite enjoyed not having his sister around (“Actually, I find her really annoying most of the time”), and there have been Xmas and birthdays to distract him. Now he will have his sick sister at home, getting lots of attention and monopolising his parents’ time to a large extent. That’s a lot to deal with when you’ve just turned 6, so we’ll be working hard to help him.

I’m going to leave it there for now. That’s enough hedging on my part on a good news day, plus I actually have a bottle of beer to drink on my own for New Year’s Eve. I hope Saff fancies sleeping through, because I might be especially hard to rouse tonight!

Have a great new year everyone. Here’s to a really good 2005 for us all.

Cheers!

Martin

T+18, T+19, T+20. The red hand and the green stool.

2004-12-29T21:38:00.000Z

Since Xmas, Saff has been exhibiting some of the classic signs of GvHD that we have been expecting. She has been a bit itchy, scratching her head during the night, so we have to massage cream into her little bald head. Her palms have been a little red and itchy, so she has a topical steroid cream. Her poo has been green (are you thinking 'Too much detail' yet?), all of which are classic signs of GvHD.

The fact is that we have been expecting GvHD to hit sometime soon, but keeping everything crossed that Saff is only affected mildly. Without wishing in any way to tempt fate, this seems to be what's happening. She doesn't seem either to be getting any infections. The only problem is that, because it is Xmas, the lab is running at minimum capacity and we are frustrated because we can't get final confirmation on any of these things. Among the top things that we're dying to know are whether there is still adenovirus in her stool (green poo can be a sign of that, as well as of GvHD) and whether it has migrated to her blood. Well, Sonja and I are known for our patience, so we'll wait. Like we have a choice!

There is some more good news. Again, this is a preliminary result due to the lab, xmas etc etc..., but the 'quick' version of another very important test shows that Saff now has 100% donor marrow. This is exactly what we want (it's what we came in for, after all!). Because this is only a preliminary result, we can't be sure that the actual result would not be 98% donor. We can, however, be sure that it won't be anything like 50%. This wait will be another test of our patience.

Saffron is off more drugs now. She is not getting any pain relief at the moment - so far so good with that one. She is also off G-CSF at the moment (this is the bone marrow production stimulant). This is routinely stopped around now to hold things back a little and hopefully mute GvHD. As a result, her neutrophil counts have fallen back from the heady heights of >5, to around 1.25. This was predicted and is not a cause for worry. Her counts could fall away to nothing again, but at that point they will reintroduce the G-CSF and confidently predict that the counts will come back. This does make us feel like we were justified in keeping our regime as tight as we did when Saffy came off green precautions. Just another dip on the rollercoaster ride!

I just wanted to mention one thing as well. Saffy's playgroup, Leapfrog, have raised over £400 from their Xmas activities on Saffy's behalf and will donate it to Great Ormond Street. That's just another example of what a great pre-school organisation it is. Really professional people, but also enormously caring. When you are in our position, you meet a lot of different people in the local health and educational support network and we hear positive comments about Leapfrog all of the time, which says a lot. Thanks very much to everyone connected to Leapfrog for the collection, GOSH is a really deserving cause. A lot of people know that the hospital is endowed in part by the proceeds from JM Barrie's Peter Pan, but this money will run out in 3 years. Believe me, if you live in the UK and - god forbid - your kids ever need anything from them, you'd want it to have at least as much resource in the future as it does now. If you can vote on company charities, wonder who to run the marathon for or whatever, please bear it in mind.

While we're on the subject, if you can do anything to support an overseas charity, please make it ISMRD (The International Society for Mannosidosis and Related Diseases) who have helped us so much. They do an incredible amount with next to no money and could achieve a great deal more with more hard currency. If you have any ideas on either score, that would make a real difference.

All the best from Team Saff.

Martin

T+16 & T + 17 - IT'S CHRISTMAS!!!!!!!!

2004-12-25T16:36:00.000Z

We’re having our fair share of the merry season here, Saff’s counts continue to rise, the number of drugs she’s taking keeps falling (Morphine and GCSF have been kicked into touch) and she seems really well. Sadly the adenovirus is still alive and kicking in her guts but this hasn’t knocked her off course to date and doesn’t seem to be troubling her doctors.

Mart and Reubs arrived on the ward with Gaby Roslin in tow this morning – marvellous. I had only just waved goodbye to the chairman and chief exec. of the hospital, wandering amongst the downtrodden to spread a little Christmas cheer. I was starting to feel like a goldfish, but lunchtime sent all visitors scurrying homeward and left the ward to its more long-term inhabitants.

Reub and Saff seemed genuinely pleased to see each other, they needed the smallest of hints to embark in a large bear hug and draw a huge AWWWW from me (and Gaby Roslin!), stood just outside.

The biggest Christmas spread you’re ever likely to see was laid out in the playroom for Fox and (close neighbour) Robin wards. Mart has already visited the buffet for two separate meals, and there are rumours it will make an appearance again tomorrow. Santa Claus (the real one you know) arrived on the ward a short while ago. Reub was delighted and Saff terrified as the white bearded jolly man arrived unannounced at her door. She cried so much she was sick and only mildly comforted by the pressie she got. Reub got a very cool transformer weapon (he wants you all to know it’s an Armada Dark Saber).

Thanks a lot for the many messages of good luck and good cheer, we’ve just been reading them here. A very happy Chrimbo to all of you taking this bumpy journey alongside us – thanks for being there!

Much love

Sonja, Martin, Reub and the Saffster

To the non-Brits:
Gaby Roslin is a minor league UK TV celeb. Actually she was quite nice - and very thin.

T +13, T +14, T +15. It's good news week.

2004-12-23T22:14:00.000Z

Saffy’s neutrophil levels continued to rise consistently over the last 2 days. She is now at a whopping 2.91. Amazing to think that just 5 days ago, she was at 0.16 and we were really encouraged by that. This is cracking news and it means that her body can fight off bacterial and fungal infections pretty much on its own now. This is what neutrophils do for a living, but they do not work against viruses, so these are still a concern.

Saff has a dose of a bug called Adenovirus in her gut at the moment. This is not a major concern as it stands and does not even require treatment. If it gets into her blood, though, that is a different story. After the ward round today, I was feeling really bullish and thinking, ‘That’s it, we’re on the home straight now’, even though I should have known better.

After the doctors left, I walked out to the kitchen and heard from another parent that their kid had been doing really well – ‘All he had was a bit of Adenovirus in his gut; we were getting ready to go home. Now it’s suddenly turned up in his blood and we’ve no idea what will happen or when’. So, back down to earth with a bit of a bump! The GvHD bogeyman is still lurking in the shadows, too, so we’ll just carry on enjoying today and see what happens tomorrow.

Speaking of which, Saffy has been (or will be) taken off 3 drugs after today’s bloods. She will not need 2 anti-fungal medications and an anti-emetic (sickness) drug. She is also moving from IV to oral Aciclovir (prophylactic anti-viral drug). This is exactly what we would have wanted at this stage.

It’s just really hard to know how to feel about things and it seems to vary wildly according to whether you are actually with Saff or not. All 3 carers tend to worry less when we’re with her. It’s strange how walking onto a hospital ward after 2 days away can feel exciting and like coming home.

Speaking of strange experiences, Xmas in hospital is just around the corner now. The ward is decorated, one of the fridges in the BMT (clean) kitchen has been requisitioned for party food, the nurses station has Xmas tunes playing most of the day and you can feel from the way the nurses are looking forward to it that it’s going to be really exciting for the kids. Saff and Reuben will both get presents delivered personally by Santa and as Saff’s counts have risen so well, we can all be in her room to open them. Unfortunately, Saffy won’t be allowed out of her room, but it should still be brilliant.

There’s talk about whether we’ll see any celebrities on Xmas day. They had Robbie Williams here one year, you know…..

Have the merriest of Xmasses everyone, here’s to a great 2005. Love from the Saffronettes.

Martin

2004-12-21T18:06:00.000Z

Saff and Baby Lucy saying a really big thank you to everyone for the hundreds of messages, cards and gifts - and all the family seconds that. Thank you so much.

T +11, T +12. Looking a gift horse in the mouth.

2004-12-21T17:55:00.000Z

The blip in Saffy's neutrophil count of 0.12, grew to 0.16. Then 0.31. And today stands at 0.53. Total white blood cell, red blood cell and platelet counts are all rising slowly.

To say that things are going well is an understatement as we should, by the law of averages, have seen infections and GvHD by now. So clearly, we're now worried that our luck cannot possibly last!

Whilst we are trying to enjoy every bit of good news when we get it, we're also trying to remain level-headed about it. For example, acute GvHD can occur up to about T +100, so we're not there yet.

I think the positive thoughts, vibes , prayers and everything else must be working!

Have to run now, I'm afraid, but wanted to share the good news and latest pictures with you all.

Love from the Saffy Squad.

Martin

2004-12-21T17:53:00.000Z

Taking advantage of not being plugged into any IV meds through the Hickman line or oral meds or food through the NG (naso-gastric) tube. I am wearing the obligatory disposable apron - you have to put a new one on every time you enter the room and throw it away as you leave.

2004-12-21T17:50:00.000Z

You might be able to make out the 2 valves in this picture. Saff is wearing a gauze 'vest' to keep the ends tidy. She is very protective her lines which are officially called 'wigglies'.

2004-12-21T17:47:00.000Z

Saff shows off her Hickman line. The rubber glove is to keep bath water away from the 2 valves that lead into her bloodstream. Hickman lines are a godsend as she has so many IV drugs, but are prone to infections - hence the precautions.

T +8, T + 9, T+ 10. Fox Ward's Star Patient

2004-12-18T20:48:00.000Z

By T +10, we were expecting to be in the throes of GvHD and fighting infections left, right and centre, but our run of good luck that seems to have followed us this year, continues.* So far.

Saffy has been on great form for the last couple of days. She is on what is regarded as minimal pain relief for this stage (slow-acting morphine), her mucositis and sore bottom have both improved enormously and she is eating and drinking. There is the first possible, tentative, do-we-dare-hope-it sign that her neutrophil count may be on the rise. Her consultant referred to her as his star patient today and it is really hard not to get your hopes up. The fact is, of course, that we can't afford to do that quite yet. Several things could be happening.

One is that the bone marrow Saffy has received has released white cells that have immediately targeted the areas like her throat and bottom and she is getting temporary relief from that. This has no relevance to engraftment of itself. She has also had red blood cell and platelet infusions recently, both of which seem to pick her up. Frankly, who knows. The safest way forward seems to be to do everything we have been doing and quietly keep fingers crossed, while expecting to run out of luck sometime soon. It is almost bound to. 80% of infections come from the body's own flora - bacteria that normally co-exist happily with us, but take their chance to multiply when the immune system is not able to respond to them. And we've always been told that GvHD is a given.

Saffy will be getting her last dose of chemo tomorrow (definitely the last one this time!). It is a final dose of methotrexate to knock the white blood cells back just a little to help counter GvHD and promote engraftment. As before, it will be followed by the Folinic Acid Rescue to start 24hrs later. And I mean 24hrs later to the minute - the accuracy makes a difference, apparently, in terms of cell regeneration cycles. The methotrexate will almost certainly make the mucositis worse, so we could be back to the no eating, no drinking, no talking routine.

If you are getting a taste for these kinds of tales, you might want to check out the site of one of our neighbours - http://www.teamhartley.co.uk - it's the site of the family whose four boys all need BMTs and are in with the second son now. Also, if this has made anyone out there think they would like to consider donating marrow, please do take 2 minutes to go to http://www.anthonynolan.org.uk/ndonors/donor.html - you really could save someone's life. And you can't say that about most things, can you?

In the meantime, may SuperSaffy's good luck continue and we can't wait to see some of you at Reuben's birthday party on Sunday. Both Sonja and I will be there.

Martin

*I wasn't counting actually hearing about the diagnosis. Obviously that didn't feel lucky at the time, but it's better than not knowing and lots has gone our way since.

2004-12-16T21:53:00.000Z

And one of Reuben looking particularly cool on one of his new presents

2004-12-16T21:20:00.000Z

Signs of the chemo starting to show, but she's still gorgeous!

2004-12-16T21:18:00.000Z

Dr Saff will see the next patient now

2004-12-16T21:14:00.000Z

Baby Lucy takes her medicine like a good girl

T +6 Time travel

2004-12-15T22:39:00.000Z

I think this is in fact T+7, so either the toing and froing has queered our pitch, or there is indeed a rip in the time-space continuum.

Seeing Saff today was brilliant and spooky in equal measure. Brilliant because I'm better and was very excited to get to the hospital. Spooky because I knew she would look completely different - but had a suspicion she would look like she did as a baby (Saff was almost entirely bald until she was two), knowing this did not prevent the shock of arriving and finding her asleep on the bed as if, in true Sleeping Beauty fashion, nothing at all had changed in the last two years. It seems we all get to dream.

Martin put paid to my reminiscence by pointing out the astonishing resemblance between Saff's remaining tufty bits and Donald Trump's ginger pate. In his defence, it was a fair call.

Saff's NG tube went in yesterday. She's unimpressed - but it means she can now be given oral medication and sufficient nutrition without too much of a battle. We've been assured that the children quickly get used to the incumbrance, and Erika already feels that this is the case.

Saff's white blood cell counts are now so low (0.02) that they haven't bothered to record the readings for neutrophils and lymphocytes, however tomorrow she is given G-CSF (granulocyte-colony stimulating factor) for the first time-which will help her to fight infection by encouraging neutrophil growth.

We're still probably at least a week away from any early signs of engraftment and the doctors and nurses are keen to point out that this will be a very tough week. We're sure they've said that at the start of every week so far though!

That's your lot I'm afraid

Take care

Sonja

T +5. Wahey!

2004-12-14T20:46:00.000Z

Today is the point on this rollercoaster ride when we stopped cranking our way to the top and just began to tip over the edge and get a glimpse of what's below. Saff is getting trouble from the mucositis in her mouth and throat and has stopped eating and barely drinks at all. She lost about 0.4kg since yesterday and her throat is so croaky that she's pretty much stopped talking too. No more 'Daddy, I happy's for a while I suspect.
Her general demeanour is best described as unimpressed and a bit grumpy and, frankly, I can't blame her. I must stress that all the problems she is getting are par for the course and no one here is at all concerned. The symptoms are among those that can be expected at this stage and it is simply a case of dealing with what presents and keeping anything from deteriorating whilst keeping Saff pain and sickness-free until her neutrophil levels start to rise. The anti-nausea meds are working and she has been moved on to oral morphine.
Saff still has raw lesions on her poor little bot. If I'd seen them on her anywhere but here I'd be going out of my mind - and so would she! Just to illustrate that GOSH is not like other hospitals (that I know, anyway) I discussed with the doctor this morning that I wasn't happy with the treatment for Saff's bottom and within 2 hours, we had a visit from the Tissue Viability Specialist Nurse who made a swift diagnosis and changed us to a different, more appropriate treatment on the spot. It felt like a visit from the Skin Problem Flying Squad. That must have been his Granada Ghia I saw parked outside. Anyway, Saffron's going to love all this open discussion of her bottom when she turns 13....
The best news of the day though, is that on Xmas day they bend the rules and Reuben can come in, meaning the 4 of us can be together. It should be a memorable Xmas, especially if Saff is out of green precautions which might just happen. Keep your fingers crossed for her.
All the best from Team Saffron.
Martin